A rare case of extensive pneumatosis cystoides intestinalis with intestinal malrotation: Case report

Intramural bowel gas (Pneumatosis intestinalis) refers to the radiological or clinical evidence of gas within the wall of the bowel lumen. While intramural gas could be secondary to life-threatening pathologies such as mesenteric ischemia in adults and necrotizing enterocolitis in neonates, it could also occur as a rare benign sub-type called Pneumatosis cystoides intestinalis, which is characterized by multiple gas-filled cysts in the submucosa and/or subserosal of the gastrointestinal tract. Distinguishing between life-threatening Pneumatosis intestinalis and its benign subtypes requires careful clinical and imaging evaluation. This involves identifying additional findings that could indicate potentially concerning causes of Pneumatosis intestinalis. Recognizing these signs is essential for effectively managing the patient because conservative management is preferred for Pneumatosis cystoides intestinalis. In this case study, we describe a patient presenting to our hospital with chronic intermittent abdominal pain persisting for about 2 years, accompanied by episodic vomiting. An abdominal CT scan revealed the presence of multiple air-filled cysts within the wall of the mal-rotated cecal bowel loop, which is abnormally located in the right upper quadrant. Associated with this pneumoperitoneum is seen in the peritoneal cavity. No other significant findings were observed on the scan. To our knowledge, this is the first case of pneumatosis cystoid interstinalis occurring in a mal-rotated gut. We also delve into the potential etiologies and management strategies for Pneumatosis cystoides intestinalis, as well as differentiating signs from the life-threatening intramural gas variant.